Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis
Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd- Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystem...
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doaj-ebd31ae573164397aec3de6a4548d0e62021-03-02T09:10:51ZengMDPI AGClinics and Practice2039-72752039-72832015-12-015310.4081/cp.2015.768321Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosisRym Ennaifer0Dhouha Bacha1Hayfa Romdhane2Myriam Cheikh3Houda Ben Nejma4Najet BelHadj5Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, TunisFaculty of Medicine, University of Tunis El Manar, Tunis; Department of Pathology, Mongi Slim Hospital, TunisDepartment of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, TunisDepartment of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, TunisDepartment of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, TunisDepartment of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, TunisSarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd- Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.https://www.clinicsandpractice.org/index.php/cp/article/view/768SarcoidosisBudd-Chiari syndromeliver. |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Rym Ennaifer Dhouha Bacha Hayfa Romdhane Myriam Cheikh Houda Ben Nejma Najet BelHadj |
spellingShingle |
Rym Ennaifer Dhouha Bacha Hayfa Romdhane Myriam Cheikh Houda Ben Nejma Najet BelHadj Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis Clinics and Practice Sarcoidosis Budd-Chiari syndrome liver. |
author_facet |
Rym Ennaifer Dhouha Bacha Hayfa Romdhane Myriam Cheikh Houda Ben Nejma Najet BelHadj |
author_sort |
Rym Ennaifer |
title |
Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
title_short |
Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
title_full |
Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
title_fullStr |
Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
title_full_unstemmed |
Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
title_sort |
budd-chiari syndrome: an unusual presentation of multisystemic sarcoidosis |
publisher |
MDPI AG |
series |
Clinics and Practice |
issn |
2039-7275 2039-7283 |
publishDate |
2015-12-01 |
description |
Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd- Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties. |
topic |
Sarcoidosis Budd-Chiari syndrome liver. |
url |
https://www.clinicsandpractice.org/index.php/cp/article/view/768 |
work_keys_str_mv |
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